Objective To investigate the differences in clinical characteristics between children and adults with systemic lupus erythematosus (SLE).Methods A retrospective cohort study was performed to investigate the differences in clinical data from 89 children and 120 adults with SLE.Clinical manifestations,laboratory results,renal pathological changes and disease activities of patients from the 2 groups were evaluated.Results The most common clinical manifestations including fever,rash,arthritis,renal damage and anemia were found in both groups.However,the incidences of symptoms such as fever,lymphadenectasis,anemia,renal damage,damage of digestive system and nerve system were higher in children with SLE than those in adult patients,and there were statistical significances (x2 =5.085,P=0.024;x2 =6.027,P =0.014;X2 =4.261,P =0.039;x2 =4.221,P =0.040;x2 =4.566,P =0.033;,x2 =4.346,P =0.037,respectively).The positive rate of antibodies against double stranded DNA in serum of children with SLE was higher than that in adults (x2 =1.895,P =0.169).However,the positive rate of antibodies against cardiolipin in serum of children with SLE was lower than that in adults,and there was statistical significance (x2 =4.823,P =0.028).Complement C3 and C4 levels in serum of children with SLE were lower than those in group of adults,and there were statistical significance (x2 =4.221,P =0.040;x2 =7.977,P =0.004,respectively).Class Ⅲ and Ⅳ were commonly observed in classification of renal pathological examinations in both groups.But it was shown that the prevalence of renal damage in children with SLE was higher than that in adult patients,and there was statistical significance (x2 =4.128,P =0.042).The most common SLE disease activity was identified as moderate in the both groups.However,the score of SLE Disease Activity Index was higher in children with SLE than that in adults,there was statistical significance (t =2.192,P =0.031).Conclusions Differences in clinical characteristics of SLE were found in children and adults.Compared with adults with SLE,children patients were found to show higher prevalence of disease activities,damage of multisystem and renal damage.Therefore,children with SLE need to be diagnosed as early as possible and treated aggressively.

To explore the characteristics of hematological abnormalities in patients with systemic lupus erythematosus (SLE) through retrospective analysis.A total of 445 SLE patients were recruited and divided into 5 groups of low white blood cell,immune thrombopenic purpura,anemia,pancytopenia and control without hematological abnormalities.Among them,351 (78.9％) had hematological abnormalities and 94 (21.1％) without hematological abnormities.The prevalence of anemia,leucocytopenia,thrombocytopenia and pancytopenia were 56.0％ (246/445),27.6％ (123/445),13.5％ (60/445) and 8.5％ (38/445) respectively.The incidence of renal damage in anemia group was significantly higher than that in control group (P ＜ 0.01).SLE patients with hematological abnormalities had lower complement C3 levels (P ＜ 0.05) and higher systemic lupus erythematosus disease activity index (SLEDAI) (P ＜ 0.01).Anemia is the most common findings in SLE patients with hematological abnormalities who have lower complement C3 levels and higher SLEDAI.

Objective To compare MRI wi th CT discography (CTD) for diagnostic assessment of lumbar disc disruption. Methods Paired comparative examination in 16 patients with ch ronic lower back pain without radicular pain and no disc herniation was conducte d using CT or MRI. The standard of CTD classification and positive disc was for mulated and the correlation between the induced lower back pain and dosage used in CTD was observed. Results For a total of 21 discs in the 16 patients, CTD showed the disc as type 2 in 12 discs and type 5 in 1 disc with 13 positive discs, while MRI only showed the high-intensity zone of poster ior annulus in 6 discs as the indirect sign of disc disruption and disc degenera tion in 7 discs. Conclusion CTD was the only method for showing the direct sign of disc disruption. The induced lower back pain was rel ated with the type of disc disruption. MRI can show some of the indirect signs of disc disruption and CTD can show the direct sign of disc disruption.

Objective To assess the perioperative change in Quality of Life(QoL)in patients who underwent CABG surgery. Methods The Chinese version of the SF-36 and SAQ were sent to participants at baseline and three and six months after CABG sur- gery.Results Angina stability score,one of the five SAQ domains,was lowest and postoperative SAQ domains scores were with sig- nificant improvement from baseline.Many of the dimensions of the SF-36 in postoperative patients were better than baseline.The SF- 36 was also used to evaluate in groups ONCAB and OPCAB,but no difference of the SF-36 subscale scores between the two groups was observed.Conclusion SAQ domains scores were significantly improved in three months and increased further in six months.Many of the dimensions of the SF-36 in postoperative patients were improved than baseline.No difference of the SF-36 subscale scores between the groups of ONCAB and OPCAB was observed postoperatively.

Objective To investigate the sexual dimorphism in the size and shape of the mandible using geometric morphometric techniques which are based on marker points,to provide a further exploration of morphological characteristics of the mandible,new ideas and theoretical foundation to support sex difference.Methods The craniofacial thin-layer CT images of 164 northern Han Chinese adults(80 males and 84 females)were used in the study,and three-dimensional coordinates of 18 landmarks were acquired.Sex differences in mandibular size and shape were assessed using the generalized procrustes superimposition,principal component analysis,and regression analysis.Results Allometric analysis showed a statistically significant effect of mandible size on shape(P<0.001),the significance of the sex differences were found in size and shape,with male mandibles larger than female mandibles,PCA plots showed considerable overlap of the male and female mandible along the extracted PCs,and logistic regression showed that the overall sex inference accuracy was 67.1%to 89.6%.Conclusion Male and female mandibles differ more in size than in shape,and when shape and size information are combined in the analysis,the accuracy of sex classification is improved to a greater extent.

OBJECTIVESWe identified the long QT syndrome (LQTS) patients, and detected the potential risk of LQTS in family members by using genetic testing and electrophysiological analysis, which helped provide clinical evaluation and appropriate treatment.

METHODSDetailed clinical characteristics and familiar history were obtained from the whole family members of an idiopathic pediatric LQTS patient. Two hundred healthy subjects with the same ethnic background were recruited as controls. The entire coding sequences of three candidate genes including KCNQ1, KCNH2 and SCN5A were screened for mutations in the proband. The function of the mutation was then explored by whole-cell patch clamp techniques, and the genetic testing and risk assessment of the family members were performed.

RESULTSThe proband was clinically preliminary diagnosed as LQTS by 12-lead electrocardiogram. On the third day of metoprolol intake (25 mg, bid), she died suddenly at lunch. One heterozygous missense mutation (SCN5A-V411M) was identified in this proband, but the mutation was absent in 200 healthy subjects. The electrophysiological analysis indicated that SCN5A-V411M significantly increased the peak current density ((230.8 ± 27.6)pA/pF vs. (101.2 ± 10.9)pA/pF, n=10, P<0.01) and the late sodium current ((156.6 ± 13.6)pA/pF vs. (95.9 ± 7.9)pA/pF, n=12, P<0.01) of sodium channel compared to wide type. The enhanced sodium channel activation with a negative shift in the peak I-V relationship was significantly higher by -50 mV than wide type (85.0%± 7.4% vs. 41.5% ± 2.6%, P<0.01), while the steady-state inactivation curves remained unchanged. Additionally, mother and grandmother of the proband were the silent mutation carriers with no symptoms, who needed the appropriate clinical assessment and follow-up. The proband's twin sister and aunt died of sudden infant death syndrome.

CONCLUSIONSWe firstly reported a heterozygote missense mutation (SCN5A-V411M) in this Chinese family. V411M induced "gain of function" of sodium channel and formed the basis of type-3 LQTS. Genetic testing could help to increase the diagnostic accuracy, and facilitate clinical assessment and appropriate therapy to prevent sudden cardiac death of individuals with SCN5A-V411M mutation.

Cardiac Conduction System Disease ; Death, Sudden, Cardiac ; Genetic Testing ; Humans ; Incidence ; Long QT Syndrome ; Mutation ; Patch-Clamp Techniques